Article
Retinoids rescue ceruloplasmin secretion and alleviate oxidative stress in Wilson's disease-specific hepatocytes.
Human molecular genetics - 28 Oct 2022
Song Dan, Takahashi Gou, Zheng Yun-Wen, Matsuo-Takasaki Mami, Li Jingyue, Takami Miho, An Yuri, Hemmi Yasuko, Miharada Natsumi, Fujioka Tsuyoshi, Noguchi Michiya, Nakajima Takashi, Saito Megumu K, Nakamura Yukio, Oda Tatsuya, Miyaoka Yuichiro, Hayashi Yohei
Abstract excerpt
Wilson's disease (WD) is a copper metabolic disorder caused by a defective ATP7B function. Conventional therapies cause severe side effects and significant variation in efficacy, according to cohort studies. Thus, exploring new therapeutic approaches to prevent progression to liver failure is urgent. To study the physiology and pathology of WD, immortalized cell lines and rodent WD models have been used...
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